Hepatic perivascular epithelioid cell tumors:The importance of preoperative diagnosis

Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.

Central Granular Cell Odontogenic Tumor: A Literature Review of Cases Reported in the Last 71 Years with a New Case Report

Central granular cell odontogenic tumors(CGCOTs)are rare,benign,slowly growing odontogenic neoplasms.Due to their uncertain histogenesis,CGCOTs are still not included as a distinct entity in the WHO classification(2017)of odontogenic tumors.We report a case of CGCOT involving the right side of maxillary anterior region of a 39-year-old white female.Immunohistochemical staining showed that granular cells positively expressed CD68 and vimentin,and negatively expressed S-100 protein.Meanwhile,we searched Pub Med,Google Scholar,and Scopus databases to summary the clinico-pathological features of 51 reported cases of CGCOT.The results showed that the granular cells of 28.6%cases were immunopositive for vimentin and CD68,and odontogenic epithelial cells were positive immunoreactivity for cytokeratin.These findings reinforced the mesenchymal origin of granular cells and the odontogenic nature of epithelium islands.

Granular cell tumor of the breast:A case report and review of literature

BACKGROUND Granular cell tumor(GCT)of the breast(GCTB)is a rare neoplasm that can exhibit malignant characteristics both clinically and radiologically.This tumor can also coexist and colocalize with breast carcinoma.CASE SUMMARY We present a patient with this uncommon tumor and discuss the diagnostic and therapeutic approaches in order to further the knowledge of GCTB and prevent misdiagnosis and overtreatment.The characteristics of the tumor,methods of diagnosis,therapy and postoperative pathological outcomes were analyzed,and relevant literatures of GCTs were reviewed.The patient underwent surgery after core needle biopsy,and the excised neoplasm was sent for pathological examination.Histological analysis revealed nests of cells with abundant pink granular cytoplasm,confirming the diagnosis of GCTB.CONCLUSION As manifestations of GCT and malignancy can mimic each other,a careful histological examination is essential before major surgery.Treatment consisting of complete excision with close clinical follow-up is recommended.

Esophageal granular cell tumors:Report of 9 cases and a literature review

Esophageal granular cell tumors(GCTs) are rare tumors of the esophagus.We evaluated the clinical and pathologic features of 9 esophageal GCT patients(5 men and 4 women) from our institute and reviewed the related disease literature.Patient age ranged from 25 to 53 years(mean:41 years).All the patients were asymptomatic or presented with non-specific symptoms.Most GCTs occurred in the distal esophagus and were less than 6 mm in diameter.Computational analysis showed that the average gray-scale endoscopic ultrasound images of esophageal GCTs were greater than that of esophageal leiomyomas.Eight patients were treated by endoscopic resection,and 1 patient underwent surgical excision.No post-therapy recurrence or metastasis developed during follow-up(mean:36.4 mo,range:1-72 mo).

Circulating tumor cells with epithelial-mesenchymal transition markers as potential biomarkers for the diagnosis of lung cancer

BACKGROUND Circulating tumor cells(CTCs) can be clustered into three subtypes according to epithelial-mesenchymal transition(EMT) markers: CTCs with epithelial markers(E-CTCs), CTCs with mesenchymal markers(M-CTCs), and CTCs with both markers(E&M-CTCs). CTC detection has clinical implications in the diagnosis of lung cancer(LC).AIM To clarify the diagnostic value of CTCs categorized by EMT markers in LC.METHODS The study included 106 patients with lung adenocarcinoma, including 42 groundglass opacities(GGO) and 64 solid lesions, who underwent surgery between July 2015 and December 2019. Eleven patients with benign tumors and seventeen healthy controls were included. CTCs in peripheral blood and associated EMT markers were detected preoperatively using the CanPatrol TM technique. The diagnostic power of CTCs for discriminating LC cases from controls was analyzed by the receiver operating characteristic(ROC) curve. The CytoploRare technique was used in 20 cases and 18 controls for validation, and Kappa values were calculated to evaluate consistency between techniques.RESULTS Of the 106 LC cases, 94(89.6%) had at least one CTC. CTCs were detectable in 35(83.3%) of 42 GGO cases. Total CTCs and E&M-CTCs were significantly more frequent in LC cases than in benign or healthy controls. The proportion of MCTCs plus E&M-CTCs increased gradually from healthy controls, to benign controls, to LC cases. The area under the ROC curve of total CTCs and E&M-CTCs was > 0.8 and > 10.75, respectively. The combined sensitivity of total-CTCs and E&M-CTCs was 85.85% for LC patients(80.95% for GGO patients) and the specificity was 78.57%.The Kappa value was 0.415,indicating relative consistency between CanPatrol TM and CytoploRare.CONCLUSION CTC detection is valuable for distinguishing LC from controls,and particularly E&M-CTC detection warrants further study.

Endoscopic resection of colorectal granular cell tumors

AIM: To determine the feasibility and effectiveness of endoscopic resection for the treatment of colorectal granular cell tumors(GCTs). METHODS: This was a retrospective study performed at a single institution. From January 2008 to April 2015, we examined a total of 11 lesions in 11 patients who were treated by an endoscopic procedure for colorectal GCTs in the Endoscopy Center, Zhongshan hospital of Fudan University, Shanghai, China. Either endoscopic mucosal resection or endoscopic submucosal dissection(ESD) was performed by three surgeons with expertise in endoscopic treatment. The pre- and postoperative condition and follow-up of these patients were evaluated by colonoscopy and endoscopic ultrasonography(EUS).RESULTS: Of these 11 lesions, 2 were located in the cecum, 3 were in the ileocecal junction, 5 were in the ascending colon, and 1 was in the rectum. The median maximum diameter of the tumors was 0.81 cm(range 0.4-1.2 cm). The en bloc rate was 100%, and the complete resection rate was 90.9%(10/11). postoperative pathology in one patient showed a tumor at the cauterization margin. however, during ESD, this lesion was removed en bloc, and no tumor tissue was seen in the wound. No perforations or delayed perforations were observed and emergency surgery was not required for complications. All patients were followed up to May 2015, and none had recurrence, metastasis, or complaints of discomfort.CONCLUSION: Endoscopic treatment performed by endoscopists with sufficient experience appears to be feasible and effective for colorectal GCTs.

Diagnosis and Treatment of a Case of Leydig Cell Tumor in Dogs

Based on such diagnostic measures as clinical diagnosis and lab etiological examination,the disease was diagnosed as Leydig cell tumor in dogs. Combined with the clinical examination results of the dog,testicular tumor removal operation was conducted,and the prognosis was favorable.

Esophageal granular cell tumors: Case report and literature review

We reported 5 cases of granular cell tumors(GCTs) of esophagus and reviewed the literature. There were 4 females and 1 male with a median age of 43 years and an average age of 44 years. All of the cases had solitary tumors. Tumor size was 0.4-2.5 cm in diameter. Gastroscopy revealed that 2 cases were located in the middle esophagus, 1 case in the upper esophagus, and 2 cases in the distal one. Five cases displayed graywhite, pink, yellow mucosal uplifts of esophagus, 3 cases had smooth surface, 1 case was slightly concave, and the biggest tumor had erosion. Tumor cells were large and polygonal with rich granular and eosinophilic cytoplasm, and small oval nuclei. Cells were arranged in nest or aciniform. Immunohistochemistry and histochemistry staining showed S-100+, neuron specific enolase+, Vim+, CD68+, smooth muscle actin-, Des-, CK-, CD117-, CD34-, Ki67-or ≤ 5%+. Periodic acidSchiff reaction and epithelial membrane antigen were both weakly positive. GCTs of esophagus are rare and most of the cases have good prognosis.

Granular cell tumor of the pancreas:A case report and review of literature

Granular cell tumors,also called Abrikossof's tumors,were originally described by Abrikossof A in 1926.The f irst case of a pancreatic granular cell tumor was described in 1975 and only 6 cases have been reported.We describe a case of granular cell tumor in the pancreas showing pancreatic duct obstruction.Because imaging studies showed f indings compatible with those of pancreatic carcinoma,the patient underwent distal pancreatectomy.Histological examination showed that the tumor consisted of a nested growth of large tumor cells with ample granular cytoplasm and small round nuclei.The tumor cells expressed S-100 protein and were stained with neuron-specific enolase and periodic acid-Schiff,but were negative for desmin,vimentin,and cytokeratin.The resected tumor was diagnosed as a granular cell tumor.To our knowledge,this is the seventh case of Granular cell tumor of the pancreas to be reported.

Multiple “Omics” data-based biomarker screening for hepatocellular carcinoma diagnosis

The huge prognostic difference between early and late stage hepatocellular carcinoma(HCC)is a challenging diagnostic problem.Alpha-fetoprotein is the mostly widely used biomarker for HCC used in the clinic,however it’s sensitivity and specificity of is not optimal.The development and application of multiple biotechnologies,including next generation sequencing,multiple“omics”data,that include genomics,epigenomics,transcriptomics,proteomics,metabolomics,metagenomics has been used for HCC diagnostic biomarker screening.Effective biomarkers/panels/models have been identified and validated at different clinical levels.A large proportion of these have a good diagnostic performance for HCC,especially for early HCC.In this article,we reviewed the various HCC biomarkers derived from“omics”data and discussed the advantages and disadvantages for diagnosis HCC.

Vulvar granular cell tumor

Granular cell tumors are rare,usually benign,soft tissue neoplasms of neural origin.They occur more often in females than males,the peak age incidence is in the fourth through fifth decades.They can occur anywhere in the body with up to 15% situated in the vulva.The commonest presentation is as an asymptomatic mass.Microscopic findings are usually sufficient,but immunohistochemistry can also be helpful in confirming the diagnosis.The vulvar tumors are benign in 98% of cases with 2% reported as malignant.In this case report we describe a woman with a granular cell tumor confirmed by biopsy who underwent excision of the mass but with focal extension to the resection margin on microscopy.Our recommendation of re-excision was declined.Since it is not uncommon with these tumors to find groups of tumor cells extending beyond the macroscopic limits of growth,we conclude that it is advisable to have margins assessed intraoperatively by frozen section such that further excision can be performed for positive margins.Our patient has been followed for 18 mo without recurrence,should the tumor recur,re-excision,with frozen section control,is indicated.Recurrence rates are reported as 2%-8% with clear margins and 20% with positive margins.

Granular cell tumor of stomach: A case report and review of literature

小粒的房间肿瘤(GCT ) 被 Abrikosoff 在 1926 第一次描述。是一个相对稀罕的瘤可以在皮或软纸巾通常发生在许多地点，而是大多数。在胃肠道的 GCT 的出现是稀罕的，财务近似为 8% 所有肿瘤，最普通的地点在之中是食管，而胃的本地化是很稀罕的。胃的 GCT 能是独居的或，更经常，与另外的胃肠的本地化联系了。尽管 GCT 通常是临床上并且组织学地良性的，一些恶意的案例被报导了。组织学地，这些肿瘤由在紧缩的“巢”和为 S-100 蛋白质的组织化学的染色支持的免疫里处理的多角形、纺锤形的房间组成从 Schwann 房间的建议推导。这个肿瘤的正确外科手术前的诊断能仅仅在 50% 所有病人被做，它总是基于内视镜的活体检视。Laparoscopic 或常规楔切除术代表选择的治疗。在这研究，作者与渗透性的模式与胃的一个独居的小粒的房间肿瘤报导了一个 49 岁的女人的一个案例，成功地与外科的切除术对待。文学的评论也有关恶意的形式在诊断标准上与强调被介绍。

Granular cell tumor of the cecum with extensive hyalinization and calcification:A case report

A granular cell tumor (GCT) is a benign neoplasm of unclear histogenesis that is generally believed to be of nerve sheath origin.GCT is not common and most often affects the tongue,skin and soft tissue,although it may occur anywhere in the body.We experienced a case of GCT that arose in the cecum of a 55-yearold man.The GCT was removed by laparoscopic resection.In addition to the tumor,endoscopic examination revealed the presence of a 5-mm-polyp in the descending colon and multiple tiny polyps in the sigmoid colon and rectum.Histological examination demonstrated a cecal tumor 1.5 cm × 1.0 cm × 0.7 cm with a hard consistency;in cut sections,mixed cells with yellowish and whitish portions were seen.The tumor was located between the mucosa and subserosa,and was composed of plump histiocyte-like tumor cells with abundant granular eosinophilic cytoplasm,which were immunoreactive for S-100 protein,vimentin,neuron-specific enolase,inhibin-α and calretinin.The tumor showed extensive hyalinization and focal dystrophic calcification.Immunohistochemical profiles did not confirm any particular cell type for the histogenetic origin of the GCT,including a nerve sheath origin.Extensive hyalinization and calcifi cation showing involution of tumor cells suggest benign clinical behavior of GCT.

Atypical granular cell tumor of the urinary bladder:A case report

BACKGROUND Granular cell tumor(GCT)is a neurogenic tumor mainly occurring in the head and neck.GCT in the genitourinary system is extremely rare and only sporadic cases of urinary bladder GCT have been reported.Most urinary bladder GCT cases are benign and only two malignant cases have been reported.Due to its rarity,no consensus criteria for the treatment of urinary bladder GCT are available at present.CASE SUMMARY A 62-year-old Chinese woman was found to have a urinary bladder tumor without any clinical manifestations on physical examination.Cystoscopy revealed a semispherical shaped lesion measuring approximately 4.0 cm in diameter at the junction of the left wall and roof of the bladder,which was covered with normal bladder mucosa.Computed tomography scan demonstrated a high-density lesion on the left wall of the bladder,measuring approximately 2.9 cm×2.4 cm with clear boundaries.Contrast-enhanced pelvic magnetic resonance imaging revealed a space-occupying lesion on the left wall of the bladder(non-mucosal origin/external pressure),which was preliminarily suspected to be a desmoplastic fibroma or leiomyoma.In the context of the above findings,a pre-operative diagnosis of bladder leiomyoma was made.The patient consequently underwent a laparoscopic partial cystectomy.The resected bladder mass looked yellowish and well-demarcated,measuring 4.0 cm×3.5 cm and infiltrated the muscular layer.The diagnosis of urinary bladder GCT was finally made by postoperative pathology,with positive immunohistochemical S-100 staining and negative pancytokeratin.The patient has been followed for 6 mo so far,with no tumor recurrence detected.CONCLUSION This case highlights the biological feature and differential diagnosis of urinary bladder GCT at the pathological and molecular levels.Transurethral resection of the bladder tumor and partial cystectomy are recommended in most urinary bladder GCT cases,while radical cystectomy is recommended in malignant cases.

Granular Cell Tumor of the Esophagus: A Patient Treated by Endoscopic Mucosal Resection with Long Term Follow-Up

Granular cell tumors (GCTs) of the esophagus are uncommon. We report a case of granular cell tumor of esophagus treated by endoscopic mucosal resection (EMR) with long term follow-up.

Granular cell tumor of the common bile duct: A Japanese case

Granular cell tumor (GCT) of the biliary system is rare. It is reported that it occurs more commonly in young black women. We report here our seldom experience of a Japanese case in whom icterus was found as a first symptom just after a caesarean operation. A 36-year-old Japanese woman developed icterus after delivery by the Caesarean operation. A surgical operation was performed without can deny that there was a tumor-related change in a bile duct as a result of examination for various images. As a result of pathological evaluation, GCT was diagnosed. By the preoperative organization biomicroscopy result, it was not able to be attachd a right diagnosis. It was thought that this tumor, although rare, should be considered as one of the causes of biliary stenosis in the younger population.

CLINICOPATHOLOGIC FEATURES AND SURGICAL TREATMENT OF NONFUNCTIONING ISLET CELL TUMORS

Objective.To in vestigate clinicopathologic characteristics and surgical results of nonfunctiona l islet cell tumors.Methods.We performed retrospective analysis of50patient s with nonfunctional islet cell tumor treat-ed at Peking Union Medical College Hospital from July1968to July1999,and summarized clinical symptoms and signs,primary diagnosis before surgery,surgical treatments,pathologic and im munohisto-logical characteristics.Results.Of the50cases examined from July 1968to July1999,12were found during physical examinations. The most common symptoms were upper abdominal pain and upper abdominal dis-com fort,which appeared in20casesand17casesrespectively,an d the most common sign was abdominal masses.B-ultrasound and CT are the most commonly used means for preoperative examination,with posit ive rates of97.8%and100%respectively.Op-e rative mortality was2%.Five-year survival rate of radical surgery of maligna nt cases was75%.Immuno-histological examinations showed that tumors rich i n multi-peptide linked hormones,neuron-specific eno-lase and chromaffin were in65%cases,90.6%and73.3% respectively.The positive rates of insulin,glucagons,somatostatin,pancreat ic polypeptide,gastrin and vasoactive intestinal peptide were68.2% ,51.2%,42.9%,40% ,25%and15.8%respectively.Conclusion.Nonfuncti onal islet cell tumors lack diagnostic specificity.Ultrasound and CT are primar y examination methods.Immunohistological analysis indicates different hormones ,but lacks the presence of related clinical symptoms.Surgery is an effective treatment for nonfunctional islet cell tumors,and even for those with distal m etastasis,immediate surgical removal and treatment can improve prognosis.

Esophageal granular cell tumor:A case report

BACKGROUND Esophageal granular cell tumor(eGCT)is a relatively rare,usually benign neoplasm of the neuroectoderm.It is derived from Schwann cells.Clinical symptoms of this disease are non-specific.However,the most common presenting symptom is dysphagia,which is mostly misdiagnosed as esophageal polyps under gastroscopy,yet it has a 2%chance of forming cancers.We report the case of a 52-year-old woman with solitary eGCT,then analysed retrospectivelyanalyze the clinical features and elucidate on the reduction of misdiagnosis and missed diagnosis.CASE SUMMARY A 52-year-old woman was diagnosed with“esophageal granulossoma”after esophagoscopy,which was mistaken for eGCT.CONCLUSION eGCT diagnosis depends on characteristic pathomorphologies and detection of the S-100 protein.Endoscopic mucosal resection is the preferred therapeutic method.

Ovarian desmoplastic small round cell tumors: Prognosis is poor! Case report

The tumor desmoplastic small round cell in women is rare. Ovarian its location is exceptional. We report a patient of 20 years, the originalNorth Africawho consulted for an increase in abdominal volume with impaired general condition. Pelvic ultrasonography and computed tomography were in favor of multiple peritoneal masses with ascites. After surgical exploration, the histological and immunohistochemical data for diagnosis small round cell desmoplastic tumor of ovarian the patient was a candidate for poly chemotherapy but she was died a month later. Diagnosis relies on histological and immunohistochemical data. The demonstration of a nonrandom translocation t (11;22) (p13;q12) is specific for the disease. The management is multidisciplinary and combining surgery, a poly aggressive chemotherapy and radiotherapy. The prognosis remains poor.

Bladder Malignant Granular Cell Tumor with EP300 Gene Mutation: A Case Report and Literature Review

Background: Malignant granular cell tumor (GCT) is extremely rare. Malignant GCT with EP300 gene mutation in the bladder has not been reported in the literature. Case Presentation: We report a special case of 45-year-old female with malignant GCT of the bladder. Pathological examination showed that the mass was 11 × 11 × 4.5 cm in size, involved in the bladder’s posterior wall. Under the microscope, the tumor cells were arranged in the shape of a nest or cord to infiltrate the bladder’s wall. The tumor cells were pleomorphic, red-stained granular within the cytoplasm, with increased nuclear/cytoplasmic ratio, vacuolar nuclei, and obvious nucleoli. The tumor cells were showed obvious nuclear atypia, and the mitosis was more than 5/50HPF. Coagulative necrosis was widely showed within the tumor. Immunohistochemistry (IHC) showed that S-100, NSE, CD68, CR, α-AT, and TFE-3 were strongly positive, and the Ki-67 proliferation index was around 15%. The next-generation high throughput sequencing indicated that EP300 gene was missense mutated (c.457A > G) with 33% mutation abundance, and genes of DPYD (c.1627A > G), ERCC1 (c.354T > C), NQO1 (c.559C > T), TPMT (c.719A > G) and XRCC1 (c.1196A > G) were polymorphic mutated. The patient died after three months of the second surgical treatment. Conclusions: We report for the first time a primary bladder malignant GCM accompanied by mutations in special driving genes such as EP300. We also conducted a comprehensive literature review and an in-depth discussion.